Stevens Johnson Syndrome (SJS) is growing in the national consciousness. Celebrities like hip-hop artist Ab-Soul, and former playmate Karen Morton have developed the syndrome. Additionally, lawmakers have declared August “Stevens Johnson Syndrome Awareness Month” in response to concerns about the illness. But what exactly is the disorder?

Autoimmune Reactions

Stevens Johnson Syndrome is a type of medical problem called an autoimmune reaction. In autoimmune reactions, the body attacks itself, using the immune system against its own tissues. In SJS, the reaction is short term, abrupt, and serious. In SJS, the body attacks its own skin, causing serious, painful damage. Stevens Johnson Syndrome can be life-threatening.

Causes of Stevens Johnson Syndrome

Many of the details of Stevens Johnson Syndrome remain elusive. Medical researchers have identified several alleles (versions of genes) that make people more likely to develop SJS, but not everyone with SJS has these genes and not everyone with these genes develops SJS.

The vast majority of people with SJS suffer the reaction in response to drugs. A wide variety of drugs have been implicated in individual cases of SJS, including epilepsy drugs, common antibiotics, and even over-the-counter pain relievers. Very rarely, bacterial, fungal and viral infections can trigger SJS. Even more rarely, cancer has been implicated. But almost all cases can be traced back to specific drugs.

Hundreds of product liability lawsuits have been filed against drug makers for failing to adequately warn about the risk of developing SJS from medications. Drug companies have paid out millions of dollars in settlements to victims and their families.

SJS Symptoms

SJS starts out with flu-like symptoms, including fever, chills, and aches. However, it rapidly progresses to a painful skin rash, with blisters, sores and ulcers. In many cases, the damage also spreads to the soft tissues of the mouth and nose. Serious cases can even spread to the surface of the internal organs and the eyes.

The most extreme cases of Stevens Johnson Syndrome progress to a more severe form of the reaction called Toxic Epidermal Necrolysis or TEN. When a patient develops TEN, the damage from SJS escalates. Patches of skin die and slough off, leaving wounds that closely resemble thermal burns. The mortality rate also jumps from the 5 to 10 percent seen in SJS to 40 percent. The mortality rate associated with TEN is higher than that associated with some strains of the Ebola virus.

SJS Treatments and Outcomes

Physicians are often at a loss on how to treat Stevens Johnson Syndrome. Steroids, which can help suppress other autoimmune diseases, may actually make SJS worse. Doctors are mostly limited to supportive therapy, treating the symptoms and making the patient comfortable until the reaction runs its course. It may take weeks for the reaction to abate.

Patients who survive SJS and TEN may suffer lifelong complications. The disorder can leave permanent, disabling and disfiguring scarring on the skin. Additionally, if the damage spread to the eyes, it may leave patients permanently blinded or sensitive to sunlight. Scarring on the internal organs can also leave patients with a lifetime of impaired organ function.

Taking Action

SJS victims and their surviving next of kin have filed lawsuits against the manufacturers of the drugs linked to their condition. These SJS lawsuits allege these drug companies were aware of the risk of SJS and failed to act on this information, endangering lives for profit.